腎上皮樣血管平滑肌脂肪瘤 ─ 案例報告及文獻回顧
顧明軒1、林子平1,2,3、鍾孝仁1,2,3、盧星華1,2,3、張延驊1,2,3、錢大維4、潘競成5、
林登龍1,2,3、陳光國1,2,3
台北榮民總醫院 泌尿部1；國立陽明大學醫學院 泌尿學科2書田泌尿科學研究中心3
台北榮民總醫院 外科部4, 台北榮民總醫院 病理部5
Epithelioid angiomyolipoma of kidney—report of nine cases and literature review
Ming-Hsuan Ku1, Tzu-Ping Lin1,2,3, Hsiao-Jen Chung1,2,3, Shing-Hwa Lu1,2,3,
Yen,-Hua Chang1,2,3, Tai-Wai Chin1, Chin-Chen Pan5, Alex Tong-Long Lin1,2,3,
Kuang-Kuo Chen1,2,3
1 Department of Urology, Taipei Veterans General Hospital;
2 School of Medicine and 3Shu-Tien Urological Institute, National Yang-Ming University, Taiwan
4 Department of Surgery, Taipei Veterans General Hospital
5Department of Pathology, Taipei Veterans General Hospital
Introduction: Several morphologic variants of Angiomyolipoma (AML) have been described in literature, including epithelioid, oncocytic, fat-predominant, smooth muscle-predominant, AML with sclerosing cysts and sclerosing types. Among these variant types of AML, epithelioid type is rare, and regarded as potentially malignant mesenchymal neoplasms. We reviewed our pathology database from January. 2008 to August. 2015, and 9 cases of epithelioid AML were found. Here we present their disease courses, and outcome.
* Abbreviations: LN, lymph node; Lt, left; Rt, right; OPN, open partial nephrectomy; ORN, open radical nephrectomy; lapa., laparoscopic ; NED, no evidence of disease; AML, angiomyolipoma; RSG, radical subtotal gastrectomy
Conclusion:
Among these 9 cases, 2 cases (22.2%) had distant metastases and ominent outcome. 3 cases with symptoms ( flank pain, palpable mass, body weight loss) were all diagnosed as malignant epithelioid AML, indicating symptomatic renal tumor may need to be managed more cautiously as potential malignancies.